Mycosis fungoides and Sézary syndrome: clinical, histopathological and immunohistochemical review and update

Thamy Yamashita, Luciana Patricia Fernandes Abbade, Mariangela Esther Alencar Marques, Silvio Alencar Marques, Thamy Yamashita, Luciana Patricia Fernandes Abbade, Mariangela Esther Alencar Marques, Silvio Alencar Marques

Abstract

This paper reviews the diagnostic and classificatory concepts of mycosis fungoides and Sézary syndrome in light of the latest normative publications. It describes the great variability of the clinical expression of mycosis fungoides in its early stages as well as the histopathological and immunohistochemical aspects that help with diagnosis. The diagnostic criteria required for characterizing Sézary syndrome and the staging system used for both mycosis fungoides and Sézary syndrome are described.

Conflict of interest statement

Conflict of interest: None

Figures

FIGURE 1
FIGURE 1
Mycosis fungoides - patch: erythematous, atrophic and slightly hyperkeratotic lesion on the gluteal region
FIGURE 2
FIGURE 2
Mycosis fungoides - patch: hypochromic and atrophic lesion on the thorax
FIGURE 3
FIGURE 3
Mycosis fungoides - plaque: erythemato-violaceous lesion with mild hyperkeratosis localized on the inguino-crural region
FIGURE 4
FIGURE 4
Mycosis fungoides: tumoral lesion appearing over a previous patch-type lesion on the leg
FIGURE 5
FIGURE 5
Mycosis fungoides: psoriasiform pattern with hyperplasia of the epidermis, band-like inflammatory infiltration and epidermotropism of lymphocytes. HE, (100X)
FIGURE 6
FIGURE 6
Mycosis fungoides: intense epidermotropism of lymphocytes on the epidermis and mild spongiosis. HE, (400X)
FIGURE 7
FIGURE 7
Mycosis fungoides: epidermotropism of lymphocytes and Pautrier’s micro-abscess. (HE X 400)
FIGURE 8
FIGURE 8
Mycosis fungoides: epidermotropism and lymphocyte alignment in the basal layer are shown. HE, (400X)
FIGURE 9
FIGURE 9
Mycosis fungoides: phenotypic studies showing CD3-positive T-cell on the epidermis and dermis. (CD3 immunostain 400X)
FIGURE 10
FIGURE 10
Mycosis fungoides: phenotypic studies showing CD4-positive T-cell on the epidermis and dermis. (CD4 immunostain 400X)

References

    1. Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105:3768–3785.
    1. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, editors. WHO classification of tumors of haematopoietic and lymphoid tissues. Lyon: IARC; 2008.
    1. Rizyl MA, Evens MST, Nelson BP, Rosen ST. T-cell non-Hodgkin lymphoma. Blood. 2008;107:1255–1284.
    1. Sanches JA, Jr, Moricz CZM, Neto CF. Processos linfoproliferativos da pele. Parte 2 - Linfomas cutâneos de células T e de células NK. An Bras Dermatol. 2006;81:7–25.
    1. Cerroni L, Gatter K, Kerl H, editors. Skin lymphoma. The illustrated guide. 3rd ed. Oxford, UK: Wiley-Blackwell; 2009. pp. 11–56. Chapter 2, Mycosis fungoides.
    1. Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, et al. Revisions to the staging and classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society of Cutaneous Lymphoma (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC) Blood. 2007;110:1713–1722.
    1. Magro CM, Crowson AN, Mihm Jr MC. The cutaneous lymphoid proliferations. A comprehensive textbook of lymphcytic infiltrates of the skin. New Jersey: Wiley- Liss; 2007. pp. 267–439.
    1. Steffen C. Ketron-Goodman disease, Woringer-Kolopp disease and pagetoid reticulosis. Am J Dermatopathol. 2005;27:68–85.
    1. Macaulay WL. Lymphomatoid papulosis. A continuing self-healing eruption, clinically bening - histologically malignant. Arch Dermatol. 1968;97:23–30.
    1. El-Shabrawi-Caelen L, Kerl H, Cerroni L. Lymphomatoid papulosis. Reappraisal of clinicopathologic presentation and classification into subtypes A, B and C. Arch Dermatol. 2004;140:441–447.
    1. Weinstock MA, Gardstein B. Twenty-year trends in the reported incidence of mycosis fungoides and associated mortality. Am J Public Health. 1999;89:1240–1244.
    1. Jenni D, Karpova MB, Seifert B, Golling P, Cozzio A, Kempf W, et al. Primary cutaneous lymphoma:two-decade comparison on a population of 263 cases from a Swiss tertiary referral centre. Br J Dermatol. 2011;164:1071–1077.
    1. Ministério da Saúde . Instituto Nacional de Câncer José Alencar Gomes da Silva (INCA) [acesso 26 jan 2012];Estimativa 2012: incidência de câncer no Brasil. Disponível em: .
    1. Sampaio SAP, Rivitti EA. Leucemias, linfomas e pseudolinfomas. In: Sampaio SAP, Rivitti EA, editors. Dermatologia. 3 ed. São Paulo: Artes Médicas; 2007. pp. 1247–1272.
    1. Pimpinelli N, Olsen EA, Santucci M, Vonderheid E, Haeffner AC, Stevens S, et al. Defining early mycosis fungoides. J Am Acad Dermatol. 2005;53:1053–1063.
    1. Burg G, Dummer R, Nestle FO, Doebbling U, Haeffner AC. Cutaneous lymphoma consist of a spectrum of nosologically different entities including mycosis fungoides and small plaque parapsoriasis. Arch Dermatol. 1996;132:567–572.
    1. Samman PD. The natural history of parapsoriasis en plaques (chronic superficial dermatitis) and prereticulotic poikiloderma. Br J Dermatol. 1972;87:405–411.
    1. Sanchez JL, Ackerman AB. The patch stage of mycosis fungoides: criteria for histologic diagnosis. Am J Dermatopathol. 1979;1:5–26.
    1. King-Ismael D, Ackerman AB. Guttate parapsoriasis/digitate dermatosis (small plaque parapsoriasis) is mycosis fungoides. Am J Dermatopathol. 1992;14:518–530.
    1. Haffner AC, Smoller BR, Zepter K, Woods GS. Differentiation and clonality of lesional lymphocytes in small plaque parapsoriasis. Arch Dermatol. 1995;131:321–324.
    1. Keehn CA, Belongie IP, Shistik G, Fenske NA, Glass LF. The diagnosis, staging and treatment options for mycosis fungoides. Cancer Control. 2007;14:102–111.
    1. Magro C, Crowson AN, Mihm MC Jr. The Cutaneous Lymphoid Proliferations. A comprehensive text book of lymphocytic infiltrates of the skin. New Jersey: John Wiley & Sons, Inc; 2007. pp. 267–299. Chapter 14, Mycosis fungoides.
    1. Hwang ST, Janik JE, Jaffe ES, Wilson WH. Mycosis fungoides and Sézary syndrome. Lancet. 2008;371:945–957.
    1. Vonderheid EC, Bernengo MG, Burg G, Duvic M, Heald P, Laroche L, et al. Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphoma. J Am Acad Dermatol. 2002;46:95–106.
    1. Sibaud V, Beylot-Barry M, Thiébaut R, Parrens M, Vergier B, Delaunay M, et al. Bone marrow histopathologic and molecular staging in epidermotropic T-cell lymphoma. Am J Clin Pathol. 2003;119:414–423.
    1. Beylot-Barry M, Vergier B. Pilotropic mycosis fungoides. J Am Acad Dermatol. 1998;38:501–502.
    1. DeBloom J 2nd, Severson J, Gaspari A, Scott G. Follicular mycosis fungoides: a case report and review of the literature. J Cutan Pathol. 2001;28:318–324.
    1. Flaig MJ, Gibson LE, Muller SA. Follicular mycosis fungoides. A histopathologic study of nine cases. J Cutan Pathol. 2001;28:525–530.
    1. Benner MF, Jansen PM, Vermeer MH, Willenze R. Prognostic factors in transformed mycosis fungoides: a retrospective analysis of 100 cases. Blood. 2012;119:1643–1649.
    1. van Doorn R, Schefter E, Willemze R. Follicular mycosis fungoides, a distinct disease entity with or without associated follicular mucinosis: a clinicopathologic abd follow-up study of 51 patients. Arch Dermatol. 2002;138:191–198.
    1. Kazakov DV, Burg G, Kempf W. Clinicopathological spectrum of mycosis fungoides. J Eur Acad Dermatol Venereol. 2004;18:397–415.
    1. Cerroni L, Fink-Punches R, Bäck B, Kerl H. Follicular mucinose: a critical reappaisal of clinicopathologic features and association with Mycosis fungoides and Sézary syndrome. Arch Dermatol. 2002;138:182–189.
    1. Gerami P, Rosen S, Kuzel T, Boone SL, Guitart J. Folliculotropic mycosis fungoides. An aggressive variant of cutaneous T-cell lymphoma. Arch Dermatol. 2008;144:738–746.
    1. Lee J, Viakhireva N, Cesca C, Lee P, Kohler S, Hoppe RT, et al. Clinicopathologic features and treatment outcomes in Woringer-Kolopp disease. J Am Acad Dermatol. 2008;59:706–712.
    1. Ackerman AB, Flaxman BA. Granulomatous mycosis fungoides. Br J Dermatol. 1970;82:397–401.
    1. Chen J, Chen G, Mao X, Li X, Li B, Lao L. Combined granulomatous slack skin/folliculotropic mycosis fungoides: case report. Int J Dermatol. 2012 [Epub ahead of print]
    1. El-Shabrawi-Calen L, Cerroni L, Medeiros LJ, McCalmont TH. Hypopigmented Mycosis Fungoides. Am J Dermatophatol. 2002;26:450–457.
    1. Ardigó M, Borroni G, Muscardin L, Kerl H, Cerroni L. Hypopigmented mycosis fungoides in caucasian patients: a clinicopathologic study of 7 cases. J Am Acad Dermatol. 2003;49:264–270.
    1. Werner B, Brown S, Ackerman B. "Hypopigmented Mycosis Fungoides" is not always mycosis fungoides. Am J Dermatopathol. 2007;27:56–67.
    1. Akaraphanth R, Douglas MC, Lim HW. Hypopigmented mycosis fungoides, treatment and a 6(1/2)-year follow-up of 9 patients. J Am Acad Dermatol. 2000;42:33–39.
    1. Lindae ML, Abel EA, Hoppe RT, Wood GS. Poikilodermatous mycosis fungoides and atrophic large-plaque parapsoriasis exhibit similar abnormalities of T-cell antigen expression. Arch Dermatol. 1988;124:366–372.
    1. Abbott RA, Sahni D, Robson A, Agar N, Whittaker S, Scarisbrick JJ. Poikilodermatous mycosis fungoides: a studyof its clinicopathological, immunophenotypic, and prognostic features. J Am Acad Dermatol. 2011;65:313–319.
    1. Georgala S, Katoulis AC, Symeonidou S, Georgala C, Vayopoulos G. Persistent pigmented purpuric eruption associated with mycosis fungoides: a case report and review of the literature. J Eur Acad Dermatol Venereol2001;15:62–64.
    1. Crowson AN, Magro CM, Zahorchack R. Atypical pigmentary purpura: a clinical, histopathologic and genotypic study. Hum Pathol. 1999;30:1004–1012.
    1. Zelger B, Sepp N, Weyrer K, Grünewald K, Zelger B. Syringotropic cutaneous T-cell lymphoma: a variant of mycosis fungoides. Br J Dermatol. 1994;130:765–769.
    1. Thein M, Ravat F, Orchard G, Calonje E, Russel-Jones R. Syringotropic cutaneous T-cell lymphoma: an immunophenotypic and genotypic study of five cases. Br J Dermatol. 2004;151:216–226.
    1. Gantcheva M, Lalova A, Brastilova V, Negenzova Z, Tsankov N. Vesicular mycosis fungoides. J Dtsch Dermatol Ges. 2005;3:898–900.
    1. Bowman PH, Hogan DJ, Sanusi ID. Mycosis fungoides bullosa: report of a case and review of the literature. J Am Acad Dermatol. 2001;45:934–939.
    1. Fenot M, Maillard H, Sierra-Fortnys S, De Ybarlucer LR, Célérier P. Syringotropic cutaneous T-cell lymphoma mimicking dermatomycosis. Ann Dermatol Venereol. 2012;139:50–53.
    1. Kodama K, Fink-Puches R, Massone C, Kerl H, Cerroni L. Papular mycosis fungoides: a new clinical variant of early mycosis fungoides. J Am Acad Dermatol. 2005;52:694–698.
    1. Requena L, Gonzales-Guerra E, Angulo J, DeVore AE, Sangueza OP. Anetodermic mycosis fungoides: a new clinico-pathological variant of mycosis fungoides. Br J Dermatol. 2008;158:157–162.
    1. Price NM, Fuks ZY, Hoffman TE. Hyperkeratotic and verrucous features of mycosis fungoides. Arch Dermatol. 1977;113:57–60.
    1. Ackerman AB, Miller RC, Shapiro L. Pustular mycosis fungoides. Arch Dermatol. 1966;93:221–225.
    1. Bergman R, Cohen A, Harth Y, Nahhas L, Shemer A, Ramon I, et al. Histopathologic findings in the clinically uninvolved skin of patients with mycosis fungoides. Am J Surg Pathol. 1995;17:452–456.
    1. Pujol RM, Gallardo F, Llistosella E, Blanco A, Bernadó L, Bordes R, et al. Invisible mycosis fungoides: a diagnostic challenge. J Am Acad Dermatol. 2000;42:324–328.
    1. Ngo JT, Trotter MJ, Haber RM. Juvenile-onset mycosis fungoides mimicking vitiligo. J Cutan Med Surg. 2009;13:230–233.
    1. van Doorn R, Van Haselen CW, van Voorst Vader PC, Geerts ML, Heule F, de Rie M, et al. Mycosis fungoides: disease evolution and prognosis of 309 Dutch patients. Arch Dermatol. 2000;136:504–510.
    1. Pileri A, Neri I, Raone B, Ciabatti S, Bellini F, Patrizi A. Mycosis fungoides following pytiriasis lichenoides: an exceptional event or a potential evolution? Pediatr Blood Cancer. 2012;58:306.
    1. Furmanczyk P, Wolgamot GM, Kussick JS, Sabath DE, Olerud JE, Argenyi ZB. Diagnosis of mycosis fungoides with different algorithmic approaches. J Cutan Pathol. 2010;37:8–14.
    1. Guitart J, Kennedy J, Ronan S, Chmiel JS, Hsiegh YC, Variakojis D. Histologic criteria for the diagnosis of mycosis fungoides: proposal for a grading system to standardize pathology reporting. J Cutan Pathol. 2001;28:174–183.
    1. Reddy K, Bhawan J. Histologic mimickers of mycosis fungoides: a review. J Cutan Pathol. 2007;34:519–525.
    1. Santucci M, Biggeri A, Feller AC, Burg G. Accuracy, concordance, and reproducibility of histologic diagnosis in cutaneous T-cell lymphoma: an EORTC Cutaneous Lymphoma Project Group Study. European Organization for Research and Treatment of Cancer. Arch Dermatol. 2000;136:497–502.
    1. Robson A. Immunocytochemistry and the dignoses of Cutaneous Lymphoma. Histopathology. 2010;56:71–90.
    1. Hodak E, David M, Maron L, Aviram A, Kaganosvsky E, Feinmesser M. CD4/CD8 double-negative epidermotropic cutaneous T-cell lymphoma: an immunohistochemical variant of mycosis fungoides. J Am Acad Dermatol. 2006;55:276–284.
    1. Fried L, Cerroni L. FOXP3 in sequencial biopsies of progressive Mycosis fungoides. Am J Dermatopathol. 2012;34:263–265.
    1. Ferrara G, Blasi AD, Zalaudek I, Argenziano G, Cerroni L. Regarding the algorithm for the diagnosis of early mycosis fungoides proposed by the International Society for Cutaneous Lymphomas: suggestions from routine histopathology practice. J Cutan Pathol. 2008;35:549–553.
    1. Baum C, Liu V. Inflammatory Mimics of Mycosis Fungoides. Pathology Case Reviews. 2011;16:45–51.
    1. Ashton-Key M, Diss TC, Du MQ, Kirkham N, Wotherspoon A, Isaacson PG. The value of the polymerase chain reaction in the diagnosis of cutaneous T-cell infiltrates. Am J Surg Pathol. 1997;21:743–747.
    1. Bergman R, Faclieru D, Sahar D, Sander CA, Kerner H, Ben-Aryeh Y, et al. Immunophenotyping and T-cell receptor gamma gene rearrangement analysis as an adjunct to the histopathologic diagnosis of mycosis fungoides. J Am Acad Dermatol. 1998;39:554–559.
    1. Knapp CF, Mathew R, Messina JL, Lien MH. CD4/CD8 dual-positive mycosis fungoides. A previously unrecognized variant. Am J Dermatopathol. 2012;34
    1. Wasco MJ, Fullen D, Su L, Ma L. The expression of MUM-1 in cutaneous T-cell lymphoproliferative disorders. Hum Pathol. 2008;39:557–563.

Source: PubMed

Sponsors et collaborateurs

Les conditions médicales

Interventions en matière de drogue

3
S'abonner